Risk factors for death of patients with cystic fibrosis awaiting lung transplantation

Rationale: The optimal timing for listing of cystic fibrosis patients for lung transplantation is controversial. Objectives: We conducted a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers to identify risk factors for death while awaiting transplantation. Methods: Data on possible risk factors were abstracted from medical records. Measurements: Time to death, patient demographic characteristics, and risk factors for death while awaiting transplantation were assessed. Univariate and multivariate survival analyses were performed using Cox regression. Results: By univariate analyses, FEV1 <= 30% predicted (HR, 3.8; 95% CI, 2.0-7.5), Pa-CO2 >= 50 mm Hg (HR, 1.85; 95% CI, 1.1-3.0), and shorter height (HR, 1.8; 95% Cl, 1.1-3.0) were associated with a higher risk of death. Referral from an accredited cystic fibrosis center was associated with a lower risk (HR, 0.53; 95% Cl, 0.30-0.92). The final multivariate model included referral from an accredited cystic fibrosis center (HR, 0.5; 95% Cl, 0.3-1.0) and listing year after 1996 (HR, 0.4; 95% Cl, 0.2-0.7); both were associated with a lower risk of death. FEV1 <= 0% predicted (HR, 6.8; 95% CI, 2.4-19.3), Pa-CO2 >= 50 mm Hg (HR, 6.9; 95% CI, 1.5-32.1), and use of a nutritional intervention (HR, 2.3; 95% Cl, 1.3-4.1) were associated with increased risk. Patients with FEV1 > 30% predicted had a higher risk of death only when their Pa-CO2 was >= 50 mm Hg (HR, 7.0; 95% Cl, 1.5-32), while the increased risk of death with FEV1 <= 30% was not further influenced by the presence of hypercapnia. Conclusions: We identified risk factors for waiting list mortality that could impact on transplant listing and allocation guidelines.